Recent data has shown that sarcoidosis, presenting initially with cardiac manifestations (CS) of either conduction system disease or cardiomyopathy and sustained VT, is not uncommon. A Canadian physician survey found that most physicians do not investigate for CS as a possibility in these situations. Thus many patients with clinically important CS are going un-diagnosed. A study from Finland showed that missing the diagnosis of CS in these patients' leads to significant mortality and morbidity.

There are no published clinical consensus guidelines on treatment of CS. Corticosteroid therapy is advocated by most experts. This is based on very modest data from small retrospective observational studies using variable definitions of clinical response. The effect of corticosteroid treatment on the clinical course of CS has not been studied in prospective studies and will be one of the aims of this project. Recent physician surveys regarding CS, in Canada and the US, found that current clinical practice varies widely. The 2008 American College of Cardiology/American Heart Association/Heart Rhythm society guidelines recommend implantation of a defibrillator (Class IIa recommendation) to prevent sudden cardiac death. The most recent Canadian device therapy guidelines do not mention CS.

A multi-center collaborative approach to study CS is greatly needed." The investigators propose exactly that i.e. a multi-center prospective cohort to start to answer clinical questions. The investigators have formed the CANADIAN CARDIAC SARCOIDOSIS RESEARCH GROUP. The group includes respirologists with an interest in sarcoidosis, cardiac electrophysiologists, cardiac imaging specialists with extensive experience in imaging of sarcoidosis and biostatisticians. The research will be in two phases and his current application is for Phase 1 (pilot) of the prospective cohort. Twenty Canadian centers have committed to participate in this pilot study.

Patients will be considered "clinically improved" if they are alive and have not had heart failure hospitalization and have not had sustained VT and one or both of: a. LV function improvement (defined as 10% decrease in LV end systolic volume) b. Resolution of conduction system disease.

Baseline assessment of Group A, B & C patients consists of: history, echocardiogram, ECG, PET +/- MRI scan(s) and QOL questionnaires. Follow-up and clinical management of Group A and B patients diagnosed with CS will occur at 6 months with: echocardiogram, ECG, QOL questionnaire, device interrogation and PET scan . Further follow-up will occur at 12, 24 and 36 months with the same parameters as measured at 6 months except PET. The timeline of follow up of Group C patients will be the same as for Groups A & B however, device interrogation will only be performed on those patients with ICD implants. Based on the planned enrollment of 400 patients, it can be estimated that 38-62 patients will have CS and will have detailed follow-up. It is anticipated that a proportion of these will be treated with steroids/immunosuppressants (use of these will be up to the treating physician discretion). The occurrence of the primary and secondary outcomes will be assessed in treated and untreated patients.

It has been suggested that newer imaging modalities (PET and CMR) may have important utility in guiding therapy of CS. FDG PET and MRI maybe able to detect the early and potentially reversible stages of CS. There will be 2 imaging core labs. The PET core lab will be located at UOHI under the direction of Dr. Robert Beanlands. The CMR core lab will be located at the Montreal Heart Institute under the direction of Dr. Mathias Friedrich. All scans will be read in the core labs by physicians who are blinded to the clinical details of the patients. All scans will be read within 24 hours of receipt and reports given to the individual centers to allow clinical decision making.

Eligibility

Ages Eligible for Study:

18 Years and older

Genders Eligible for Study:

Both

Accepts Healthy Volunteers:

No

Sampling Method:

Probability Sample

Study Population

Electrophysiology Service patients

Criteria

Inclusion Criteria:

Group A: < 60 with unexplained, new onset, significant conduction system disease, to screen for CS as underlying etiology Group B: idiopathic non-ischemic dilated cardiomyopathy and sustained VT, to screen for CS as underlying etiology Group C: diagnosed with pulmonary/systemic sarcoidosis and being screened for CS (biopsy proven extra-cardiac sarcoidosis and abnormal ECG

Exclusion Criteria:

unable or unwilling to provide informed consent

patients who are pregnant or lactating

patients with known claustrophobia

age < 18 years

Contacts and Locations

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To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below.
For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT01477359